Morquio syndrome (morquio-brailsford syndrome; brailsford-morquio dystrophy; familial osseous dystrophy; keratosulfaturia; mps iv; mucopolysaccharidosis iv; spondyloepiphyseal dysplasia; osteochondrodystrophia deformans; infantile hereditary chondrodysplasia; hereditary poly topic enchondral dysostosis; hereditary osteochondrodystrophy; eccentro-osteochondrodysplasia; dysostosis enchondralis meta- epiphysaria; morquio-ullrich syndrome; atypical chondrodystrophy; chondrodystrophia tarda; chondro-osteodystrophy)

General: Autosomal recessive dystrophy of cartilage and bone; slight predilection for males; apparent between ages 4 and 10 years; excess production of keratosulfate (see Hurler Syndrome; Hunter Syndrome; Sanfilippo-Good Syndrome; Scheie Syndrome; Maroteaux-Lamy Syndrome); autosomal recessive; abnormal N-acetylgalactosamine-G-sulfate sulfatase.

Ocular: Enophthalmos; ptosis; excessive tear secretion; ocular hypotony; miosis; occasionally hazy cornea; bushy eyebrows; optic nerve atrophy; moderate-to-late corneal clouding.

Clinical: Dwarfism; skeletal deformities (progressive); delayed ossification of epiphyses; decreased muscle tone; deafness; weak extremities; waddling gait; coarse broad mouth; spaced teeth; aortic regurgitation; normal intelligence.

Fraunfelder FT, Roy FH. Current Ocular Therapy. 5th ed. Philadelphia: WB Saunders. 2000.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Kenyon KR, et al. The systemic mucopolysaccharidoses: ultrastructural and histochemical studies of conjunctiva and skin. Am J Ophthalmol 1972; 73:811.

MacDessi JJ, et al. Spondylo-epiphyseal dysplasia with ocular changes: report of two 'new' variants in two different families. Pediatr Radiol 1978; 7:22G-228.

Morquio L. Sur une Forme de Dystrophie Osseuse Familiale. Bull Soc Pediatr (Paris) 1929; 27:145-l52.